Malignant Disease Flashcards
Most common childhood cancers
Leukaemia followed by brain and spinal tumours
Then lymphomas
Ages incidence of leukaemia
Affects all ages but there is an early childhood peak
When are neuroblastoma and Wilms tumour seen
Almost always seen before 6 years old
When are Hodgkin lymphoma and bone tumours most seen?
Peak incidence in adolescence and early adult life
5 year survival of all children with cancers
75%
Which syndromes are associated with an increased risk of cancer?
Down syndrome associate with leukaemia
Neurofibromatosis associated with glioma
What can be used to identify bone marrow disease?
Nuclear medicine imaging eg. Radio labelled technetium bone scan
What is a tumour marker for neuroblastoma?
Urinary catecholamine excretion
What is tumour marker of germ cell tumours and liver tumours?
High alphafetoprotein production
Which age of childhood cancer has poorest prognosis?
Teenagers and young adults have poorer outcomes than children
Role of radiotherapy in childhood cancers
Retains a role in treatment of some tumours but the risk of damage to growth and function of normal tissues is higher than in adults
Therefore need to protect organs and tissues
Also keeping a child still is more difficult
When can bone marrow be useful in childhood cancers and when are the various types used?
Allogenic transplants (from a compatible donor) of stem cells is principally used for high risk or relapsed leukaemia Autologous (from patient harvested beforehand) are most commonly used for children whose prognosis is poor when use high dose chemotherapy
What is the big risk in children with cancer?
Risk of serious infection due to being immunocompromised as a result of chemo and also the cancer
Therefore need to monitor for fever and neutropenia - if either then antibiotic treatment started
Which opportunistic infections are associated with cancer therapy? x3
PCP (especially in leukaemia patients) and disseminated fungal infections (aspergillosis and candidiasis)
Also coagulate-negative staph infections from CVC’s
Which viral infections are worse in cancer patient than a healthy child? x2
Chickenpox and measles can be life threatening
Other viral infections are no worse
Immunoglobulin for at risk children
GIT problems in children with cancer x3
Mouth ulcers are common therefore feeding is painful
Also chemotherapy agents are nauseating and induce vomiting
Therefore often do not feed well
Also chemotherapy induced but mucosal damage cause diarrhoea and may predispose to gram negative infection
What can doxorubicin cause?
Cardio toxicity
What can cyclophosphamide cause?
Haemorrhagic cystitis
What can cisplatin cause?
Renal failure and deafness
What can vincristine cause?
Neuropathy
Which is the most common leukaemia in children
Acute lymphoblastic leukaemia - accounts for 80% of all leukaemias in children
Peak age of presentation of ALL
2-5 years
How does ALL present? x10
Clinical symptoms and signs are from disseminated disease and systemic ill health from infiltration of bone marrow or other organs
Therefore malaise, anorexia, anaemia (pallor and lethargy), neutropenia (infections), thrombocytopenia (bruising, petechiae, nose bleeds), bone pain from infiltration
Hepatosplenomegaly or lymphadenopathy
CNS signs
Testicular enlargement
Onset of ALL normally
Normal presents insidiously over several weeks - but can progress very quickly
What will FBC show in ALL
Abnormal in most children, low hb, thrombocytopenia, evidence of circulating leukaemic blast cells
What investigation needs to be done to confirm ALL?
Bone marrow examination - essential to identify it but also to provide prognostic information
What other examination should be done in ALL?
Chest X-ray to look for mediastinal mass - characteristic of T cell disease
What needs to be done before ALL treatment can begin? X4
Correct anaemia (blood transfusion), decrease risk of bleeding by giving platelets, treat any infection Allopurinol (or urate oxidase) when risk is high and high white cell count - to protect renal function against effects of rapid cell lysis
Treatment of ALL
4 weeks of combined chemotherapy - vincristine, steroid, methotrexate intrathecally (normal chemo doesn’t cross BBB)
Followed by 3 weeks consolidation (first 3 + thiopurine)
Then monthly maintenance vincristine + steroids, daily 6mcp and weekly methotrexate orally - for up to 3 years
Also delayed intensification at 16weeks to consolidate remission - lots of drugs
What else is given to ALL patients
Co-trimoxazole prophylaxis against PCP
Maintenance of remission with current treatment in ALL
Remission in 95%
What sort of brain tumours do children get?
Almost always primary and 60% infratentorial (cerebellum)
Most common child brain tumours x2
Astrocytoma (40%) - varies from benign to highly malignant
Medulloblastoma (20%) midline of posterior fossa and 20% have spinal mets at diagnosis
Presentation of brain tumour in child
Often due to raised ICP but can be focal neurological signs
Detected depending on site
Management of brain tumour x3
MRI
Surgery aiming at treating hydrocephalus and providing tissue diagnosis (biopsy) and doing maximum resection
(if possible - eg. not in brain stem)
Radiotherapy and chemo vary with tumour type and age of patient
Which lymphomas are most common when?
Hodgkin most common in adolescence
Non Hodgkin most common in childhood
How does Hodgkin lymphoma present? x4
Mostly painless lymphadenopathy
Can cause airway obstruction
Often long history (months) and
the systemic symptoms eg. sweating, pruritus, weight loss and fever are uncommon
Investigation of Hodgkin lymphoma x3
Lymph node biopsy, imaging of all nodal sites and bone marrow biopsy
Management of Hodgkin lymphoma
Chemo with or without radio
PET scan used to monitor treatment response
Prognosis of Hodgkin lymphoma
Overall 80% can be cured and even disseminated disease 60% can be cured
Prognosis of non Hodgkin lymphoma
Survival rates over 80% for B and T cell disease
What is a neuroblastoma?
Arise from neural crest tissue in adrenal medulla and sympathetic nervous system
When is neuroblastoma most common?
Before age of 5
How do most children with neuroblastoma present?
Most present with abdominal mass but primary tumour can lie anywhere along sympathetic chain (from neck to pelvis)
Can be large and complex mass (enveloping major blood vessels and lymph nodes)
Also get pallor, weight loss, hepatomegaly (met), bone pain or limp (mets)
What do children with neuroblastoma over 2 usually present with
Clinical symptoms from metastatic disease
Investigations in neuroblastoma
Clinical and radiological features
Also raised catecholamine secretion
Confirmatory biopsy
Prognosis of neuroblastoma
In very young infants - can spontaneously regress
Majority of children over 1 have advanced disease and poor prognosis
Treatment of neuroblastoma
Local primary - surgery
Mets with chemotherapy high dose and autologous bone marrow transplant
Relapse rate is high and cure rate low (30%) with metastatic disease
What is Wilms Tumour?
Nephroblastoma - embryonical renal tissue tumour - commonest renal tumour of childhood
When do you see Wilms tumour?
80% before age 5 and rarely after 10
Presentation of Wilms
Large abdominal mass usually found incidentally in otherwise well child
Can also uncommonly have abdominal pain, anorexia, anaemia (haemorrhage into mass), haematuria, hypertension
Treatment of Wilms
Initial chemo and then nephrectomy - tumour then staged histologically and treatment decided on - radio restricted to advanced disease
Prognosis for Wilms
Good with over 80% cure rate
If present with mets 60% cure rate
Most common soft tissue sarcoma in children
Rhabdomyosarcoma (develop from skeletal muscle cells)
Wide variety of sites therefore varying presentation and prognosis
Most common sites for rhabdomyosarcoma
Head and neck - 40% causing local signs
When are bone tumours found
Uncommon before puberty
Osteogenic sarcoma more common than Ewing but Ewing more in younger children
Gender predominance of bone tumours
Male predominance
Common sites for bone tumours
Limbs most common site and bone pain most common feature - otherwise well
Investigation of bone tumour x4
Plain X-ray for detection followed by MRI and bone scan
Chest CT to look for lung mets
Treatment of bone tumours
Chemo before surgery - avoid amputation if possible - endoprosthetic resection of bone and replacement
Presentation of retinoblastoma
Most within first 3 years of life with white pupillary reflex (not red) or with squint
Treatment of retinoblastoma x3
Chemo and then laser therapy to retina
Radio in advanced disease
Incidence of cancer before age of 15
1 in 500
Inheritance of retinoblastoma
All bilateral are hereditary and about 20% of unilateral
Dominant inheritance but with incomplete penetrance
Susceptibility gene is on chromosome 13
Prognosis of retinoblastoma
Most are cured but many are visually impaired
